Hair Loss in Lichen Planopilaris and Frontal Fibrosing Alopecia: Not Always Irreversible
SKIN APPENDAGE DISORDERS
Authors: Batra, Prag; Sukhdeo, Kumar; Shapiro, Jerry
Abstract
Introduction: We present 2 cases in which typically irreversible lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) showed signs of reversal. Case Presentation: A 27-year-old Caucasian man presented with hair loss and intense pruritus on the vertex scalp for 4 years with biopsy-proven LPP and having failed multiple pharmacologic modalities. Six months after adding oral tofacitinib and later dapsone, he demonstrated reduced scalp visibility, evidence of crown and vertex hair regrowth, and elimination of itch. A 45-year-old premenopausal Hispanic woman presented with eyebrow loss for 3.75 years and hair loss for 9 months with biopsy-proven FFA. After beginning oral finasteride and hydroxychloroquine, triamcinolone injections, and topical minoxidil, she initially worsened over 11 months but subsequently improved over 6 months, demonstrating hair and eyebrow regrowth, reduction in glabella-hairline distance, and new absence of frontal hair line hyperkeratosis and inflammation. Discussion/Conclusion: Cicatricial alopecia involves inflammation with JAK-STAT upregulation. We report a positive clinical response in LPP to tofacitinib, a JAK1/3 inhibitor, and dapsone, an anti-neutrophilic agent. FFA is believed to involve autoimmune and/or hormonal processes. Here we report a positive clinical response to androgenic and immune modulators.
Right ethmoid eosinophilic angiocentric fibrosis with orbital extension
EUROPEAN ANNALS OF OTORHINOLARYNGOLOGY-HEAD AND NECK DISEASES
Authors: Gorostis, S.; Bacha, M.; Gravier, S.; Raguin, T.
Abstract
Introduction: Eosinophilic angiocentric fibrosis (EAF) is a slowly progressive, benign disease involving the mucosa of the upper airways or, more rarely, the orbit. It belongs to the spectrum of IgG4-related disease. Case report: The authors report the case of a 61-year-old man who presented with orbital involvement (visual loss, pain, proptosis, and eyelid oedema), headache and nasal obstruction. Imaging revealed a right ethmoido-orbital mass infiltrating the periorbital fat and enveloping the optic nerve. Histological examination concluded on a diagnosis of EAF in the presence of perivascular infiltration by inflammatory cells, predominantly eosinophils, and zones of "onion skin" fibrosis. Immunohistochemistry attributed these lesions to IgG4-related disease. Initial treatment with corticosteroids followed by dapsone failed to control the disease and resulted in severe steroid dependence. Surgical ethmoidectomy with resection of the lamina papyracea was performed to allow displacement of the eyeball into the nasal cavity in the event of another episode. Discussion: The combination of surgery and rituximab achieved lasting pain relief with no recurrence of exophthalmos. Copyright (C) 2017. Published by Elsevier Masson SAS.